| Distribution of ubiquilin 2 and TDP-43 aggregates throughout the CNS in UBQLN2 p.T487I-linked amyotrophic lateral sclerosis and frontotemporal dementia. | Distribution of ubiquilin 2 and TDP-43 aggregates throughout the CNS in UBQLN2 p.T487I-linked amyotrophic lateral sclerosis and frontotemporal dementia.
Nementzik LR, Thumbadoo KM, Murray HC, Gordon D, Yang S, Blair IP, Turner C, Faull RLM, Curtis MA, McLean C, Nicholson GA, Swanson MEV, Scotter EL., Free PMC Article | 04/24/2024 |
| Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways. | Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways.
Idera A, Sharkey LM, Kurauchi Y, Kadoyama K, Paulson HL, Katsuki H, Seki T. | 06/3/2023 |
| UBQLN2 restrains the domesticated retrotransposon PEG10 to maintain neuronal health in ALS. | UBQLN2 restrains the domesticated retrotransposon PEG10 to maintain neuronal health in ALS.
Black HH, Hanson JL, Roberts JE, Leslie SN, Campodonico W, Ebmeier CC, Holling GA, Tay JW, Matthews AM, Ung E, Lau CI, Whiteley AM., Free PMC Article | 04/7/2023 |
| UBQLN2 undergoes a reversible temperature-induced conformational switch that regulates binding with HSPA1B: ALS/FTD mutations cripple the switch but do not destroy HSPA1B binding. | UBQLN2 undergoes a reversible temperature-induced conformational switch that regulates binding with HSPA1B: ALS/FTD mutations cripple the switch but do not destroy HSPA1B binding.
Phung TH, Tatman M, Monteiro MJ., Free PMC Article | 01/7/2023 |
| Damaged DNA Is an Early Event of Neurodegeneration in Induced Pluripotent Stem Cell-Derived Motoneurons with UBQLN2(P497H) Mutation. | Damaged DNA Is an Early Event of Neurodegeneration in Induced Pluripotent Stem Cell-Derived Motoneurons with UBQLN2(P497H) Mutation.
Zhang Y, Zeng B, Gu A, Kang Q, Zhao M, Peng G, Zhou M, Liu W, Liu M, Ding L, Liang D, Liu X, Liu M., Free PMC Article | 10/22/2022 |
| Multi-omics profiling identifies a deregulated FUS-MAP1B axis in ALS/FTD-associated UBQLN2 mutants. | Multi-omics profiling identifies a deregulated FUS-MAP1B axis in ALS/FTD-associated UBQLN2 mutants.
Strohm L, Hu Z, Suk Y, Rühmkorf A, Sternburg E, Gattringer V, Riemenschneider H, Berutti R, Graf E, Weishaupt JH, Brill MS, Harbauer AB, Dormann D, Dengjel J, Edbauer D, Behrends C., Free PMC Article | 07/16/2022 |
| Amyotrophic lateral sclerosis (ALS) linked mutation in Ubiquilin 2 affects stress granule assembly via TIA-1. | Amyotrophic lateral sclerosis (ALS) linked mutation in Ubiquilin 2 affects stress granule assembly via TIA-1.
Peng G, Gu A, Niu H, Chen L, Chen Y, Zhou M, Zhang Y, Liu J, Cai L, Liang D, Liu X, Liu M., Free PMC Article | 05/14/2022 |
| Towards a molecular understanding of the overlapping and distinct roles of UBQLN1 and UBQLN2 in lung cancer progression and metastasis. | Towards a molecular understanding of the overlapping and distinct roles of UBQLN1 and UBQLN2 in lung cancer progression and metastasis.
Shah PP, Saurabh K, Kurlawala Z, Vega AA, Siskind LJ, Beverly LJ., Free PMC Article | 04/16/2022 |
| ALS/FTD mutations in UBQLN2 are linked to mitochondrial dysfunction through loss-of-function in mitochondrial protein import. | ALS/FTD mutations in UBQLN2 are linked to mitochondrial dysfunction through loss-of-function in mitochondrial protein import.
Lin BC, Phung TH, Higgins NR, Greenslade JE, Prado MA, Finley D, Karbowski M, Polster BM, Monteiro MJ., Free PMC Article | 04/2/2022 |
| Human Ubiquilin 2 and TDP-43 copathology drives neurodegeneration in transgenic Caenorhabditis elegans. | Human Ubiquilin 2 and TDP-43 copathology drives neurodegeneration in transgenic Caenorhabditis elegans.
Saxton AD, Kraemer BC., Free PMC Article | 01/8/2022 |
| Previously uncharacterized interactions between the folded and intrinsically disordered domains impart asymmetric effects on UBQLN2 phase separation. | Previously uncharacterized interactions between the folded and intrinsically disordered domains impart asymmetric effects on UBQLN2 phase separation.
Zheng T, Galagedera SKK, Castañeda CA., Free PMC Article | 12/25/2021 |
| ALS-linked mutations impair UBQLN2 stress-induced biomolecular condensate assembly in cells. | ALS-linked mutations impair UBQLN2 stress-induced biomolecular condensate assembly in cells.
Riley JF, Fioramonti PJ, Rusnock AK, Hehnly H, Castañeda CA., Free PMC Article | 11/22/2021 |
| Kinetic Constraints in the Specific Interaction between Phosphorylated Ubiquitin and Proteasomal Shuttle Factors. | Kinetic Constraints in the Specific Interaction between Phosphorylated Ubiquitin and Proteasomal Shuttle Factors.
Qin LY, Gong Z, Liu K, Dong X, Tang C., Free PMC Article | 09/25/2021 |
| Global proteomics of Ubqln2-based murine models of ALS. | Global proteomics of Ubqln2-based murine models of ALS.
Whiteley AM, Prado MA, de Poot SAH, Paulo JA, Ashton M, Dominguez S, Weber M, Ngu H, Szpyt J, Jedrychowski MP, Easton A, Gygi SP, Kurz T, Monteiro MJ, Brown EJ, Finley D., Free PMC Article | 08/28/2021 |
| UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model. | UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model.
Zhang K, Wang A, Zhong K, Qi S, Wei C, Shu X, Tu WY, Xu W, Xia C, Xiao Y, Chen A, Bai L, Zhang J, Luo B, Wang W, Shen C. | 07/24/2021 |
| Prognostic and predicted significance of Ubqln2 in patients with hepatocellular carcinoma. | Prognostic and predicted significance of Ubqln2 in patients with hepatocellular carcinoma.
Luo YD, Yu HQ, Liu XY, Huang D, Dai HS, Fang L, Zhang YJ, Lai JJ, Jiang Y, Shuai L, Zhang LD, Chen G, Bie P, Xie CM., Free PMC Article | 05/22/2021 |
| [Association between rare UBQLN2 variants and amyotrophic lateral sclerosis in Chinese population]. | [Association between rare UBQLN2 variants and amyotrophic lateral sclerosis in Chinese population].
Chen JY, Liu XY, Xu YS, Fan DS. | 04/13/2021 |
| UBQLN2 Promotes the Production of Type I Interferon via the TBK1-IRF3 Pathway. | UBQLN2 Promotes the Production of Type I Interferon via the TBK1-IRF3 Pathway.
Chen T, Zhang W, Huang B, Chen X, Huang C., Free PMC Article | 02/27/2021 |
| ALS/FTD mutations in UBQLN2 impede autophagy by reducing autophagosome acidification through loss of function. | ALS/FTD mutations in UBQLN2 impede autophagy by reducing autophagosome acidification through loss of function.
Wu JJ, Cai A, Greenslade JE, Higgins NR, Fan C, Le NTT, Tatman M, Whiteley AM, Prado MA, Dieriks BV, Curtis MA, Shaw CE, Siddique T, Faull RLM, Scotter EL, Finley D, Monteiro MJ., Free PMC Article | 10/24/2020 |
| Findings suggest that the changes in physical properties caused by ALS-linked Pxx mutations modify ubiquilin 2 protein (UBQLN2) behavior in vivo. | ALS-Linked Mutations Affect UBQLN2 Oligomerization and Phase Separation in a Position- and Amino Acid-Dependent Manner.
Dao TP, Martyniak B, Canning AJ, Lei Y, Colicino EG, Cosgrove MS, Hehnly H, Castañeda CA., Free PMC Article | 05/2/2020 |
| UBQLN2 UBL is fine-tuned for the hRpn10 UIM-1 site. | Structure of hRpn10 Bound to UBQLN2 UBL Illustrates Basis for Complementarity between Shuttle Factors and Substrates at the Proteasome.
Chen X, Ebelle DL, Wright BJ, Sridharan V, Hooper E, Walters KJ., Free PMC Article | 03/7/2020 |
| Its mutation is found in neurodegenerative diseases like amyotrophic lateral sclerosis, spastic paraplegia, and frontotemporal dementia.( | Striking phenotypic variation in a family with the P506S UBQLN2 mutation including amyotrophic lateral sclerosis, spastic paraplegia, and frontotemporal dementia.
Gkazi SA, Troakes C, Topp S, Miller JW, Vance CA, Sreedharan J, Al-Chalabi A, Kirby J, Shaw PJ, Al-Sarraj S, King A, Smith BN, Shaw CE. | 12/21/2019 |
| our data highlight a safeguarding function of the MTM1-UBQLN2 complex that ensures cytoskeletal integrity to avoid proteotoxic aggregate formation | The MTM1-UBQLN2-HSP complex mediates degradation of misfolded intermediate filaments in skeletal muscle.
Gavriilidis C, Laredj L, Solinhac R, Messaddeq N, Viaud J, Laporte J, Sumara I, Hnia K. | 04/13/2019 |
| These results reveal a previously unrecognized role for UBQLN2 in regulating the early stages of liquid-liquid phase separation by directly modulating the fluidity of protein-RNA complexes and the dynamics of SG formation. | Ubiquilin 2 modulates ALS/FTD-linked FUS-RNA complex dynamics and stress granule formation.
Alexander EJ, Ghanbari Niaki A, Zhang T, Sarkar J, Liu Y, Nirujogi RS, Pandey A, Myong S, Wang J., Free PMC Article | 02/9/2019 |
| polyQ-expanded Htt-N552 and Atx-3 sequester endogenous Ub adaptors, human RAD23 homolog B (hHR23B) and ubiquilin (UBQLN)-2, into inclusions. This sequestration effect is dependent on the UBA domains of Ub adaptors and the conjugated Ub of the aggregated proteins. | PolyQ-expanded huntingtin and ataxin-3 sequester ubiquitin adaptors hHR23B and UBQLN2 into aggregates via conjugated ubiquitin.
Yang H, Yue HW, He WT, Hong JY, Jiang LL, Hu HY. | 01/19/2019 |